Sohal Aalam, Kowdley Kris V
Liver Institute Northwest, Seattle, WA.
Elson Floyd College of Medicine, Spokane, WA.
ACG Case Rep J. 2024 Jan 16;11(1):e01256. doi: 10.14309/crj.0000000000001256. eCollection 2024 Jan.
Primary sclerosing cholangitis (PSC) is a progressive cholestatic liver disease characterized by intrahepatic and extrahepatic bile duct strictures leading to cirrhosis. A subtype with elevated serum immunoglobulin (Ig) G4 levels has been recently identified. Elevated IgG4 titers can be present in 9%-15% of patients with PSC. Currently, liver transplantation is the only effective treatment of PSC, although multiple medical therapies are under evaluation. We report a case of a young adult with PSC and elevated IgG4 levels who had marked serum aminotransferase elevation; the patient had an incomplete response to steroids but achieved complete biochemical remission after initiation of oral vancomycin.
原发性硬化性胆管炎（PSC）是一种进行性胆汁淤积性肝病，其特征为肝内和肝外胆管狭窄，最终导致肝硬化。最近发现了一种血清免疫球蛋白（Ig）G4水平升高的亚型。9%-15%的PSC患者可出现IgG4滴度升高。目前，肝移植是PSC唯一有效的治疗方法，不过多种药物治疗正在评估中。我们报告一例年轻的PSC成年患者，其IgG4水平升高，血清氨基转移酶显著升高；该患者对类固醇治疗反应不完全，但在开始口服万古霉素后实现了完全生化缓解。
